Successful treatment of progressive diffuse PEComatosis

Diffuse PEComatosis. a) Many of the nodules comprise cytologically bland, clear cells, while b) focally, the cells show a more spindled morphology with the nuclei being blunt-ended and containing moderate volumes of eosinophilic cytoplasm characteristic of lymphangioleiomyomatosis. Haematoxylin and eosin. Magnification: ×200 c) Computed tomography (CT) 18 months after diagnosis showed marked interlobular septal thickening in the upper lobes, a probable micronodular pattern (on this thick CT section) and a right-sided pleural effusion, but no cystic air spaces. d) 3 months later there was an improvement with less thickening of the interlobular septa (a micronodular pattern persists) and almost complete resolution of the right pleural effusion.